Alopecia ; chemically induced ; diagnosis ; Antirheumatic Agents ; administration & dosage ; adverse effects ; Arthritis, Rheumatoid ; drug therapy ; Biopsy ; methods ; Cyclosporine ; administration & dosage ; Dermatologic Agents ; administration & dosage ; Drug Hypersensitivity Syndrome ; diagnosis ; etiology ; physiopathology ; therapy ; Humans ; Male ; Middle Aged ; Prednisolone ; administration & dosage ; Skin ; pathology ; Sulfasalazine ; administration & dosage ; adverse effects ; Symptom Flare Up ; Treatment Outcome ; Vitiligo ; chemically induced ; diagnosis

Adult ; Humans ; Male ; Pityriasis Lichenoides ; diagnosis ; drug therapy ; immunology ; Prednisolone ; administration & dosage ; therapeutic use ; Psoriasis ; diagnosis ; drug therapy ; immunology ; Young Adult

Toxic epidermal necrolysis (TEN) is a rare, acute, serious, and potentially fatal skin disease, in which cell death causes the epidermis to separate from the dermis. It is thought to be a hypersensitivity complex that affects the skin and mucous membranes, and is caused by certain medications, infections, genetic factors, underlying immunologic disease, or more rarely, cancers. We report two cases of TEN associated with deflazacort (DFZ), a derivative of prednisolone, used in the first episode of nephrotic syndrome (NS). The skin eruption appeared on the 4(th) and 5(th) weeks after DFZ administration, while NS was in remission. The widespread lesions were managed by intensive supportive treatment, discontinuation of DFZ, and oral administration of cyclosporine. Both patients showed a rapid improvement in symptoms of TEN without any complications or relapse of NS.
Administration, Oral ; Cell Death ; Cyclosporine* ; Dermis ; Epidermis ; Humans ; Hypersensitivity ; Immune System Diseases ; Mucous Membrane ; Nephrotic Syndrome* ; Prednisolone ; Recurrence ; Skin ; Skin Diseases ; Stevens-Johnson Syndrome*

Entecavir (Baraclude®) is an oral antiviral drug used for the treatment of HBV. Entecavir is a reverse transcriptase inhibitor which prevents the HBV from multiplying. Most common adverse reactions caused by entecavir are headache, fatigue, dizziness, and nausea. Until now, there has been no report of peripheral neuropathy as a side effect associated with entecavir treatment. Herein, we report a case of peripheral neuropathy which probably occurred after treatment with entecavir in a hepatitis B patient. The possibility of the occurrence of this side effect should be carefully taken into consideration when a patient takes a high dose of entecavir for a long period of time or has risk factors for neuropathy at the time of initiating entecavir therapy.
Administration, Oral ; Antiviral Agents/*adverse effects/therapeutic use ; Brain/diagnostic imaging ; Drug Therapy, Combination ; Duloxetine Hydrochloride/therapeutic use ; Glucocorticoids/therapeutic use ; Guanine/adverse effects/*analogs & derivatives/therapeutic use ; Hepatitis B, Chronic/drug therapy ; Humans ; Male ; Middle Aged ; Polyneuropathies/*diagnosis/drug therapy/etiology ; Prednisolone/therapeutic use ; Pregabalin/therapeutic use ; Tomography, X-Ray Computed

We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.
Administration, Oral ; Bronchoconstriction ; Chin ; Female ; Heart Failure ; Hepatomegaly ; Humans ; Hypoglycemia ; Hypotension ; Hypothyroidism ; Liver ; Prednisolone ; Propranolol ; Psychomotor Agitation ; Thigh ; Thyroid Function Tests ; Thyroid Gland ; Thyroxine

No abstract available.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Carotid Artery Diseases/*complications/diagnosis/therapy ; Cerebral Angiography/methods ; Diagnosis, Differential ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hydrocortisone/therapeutic use ; Hypopituitarism/diagnosis/*etiology/therapy ; Intracranial Aneurysm/*complications/diagnosis/therapy ; Magnetic Resonance Imaging ; Plasma Substitutes/administration & dosage ; Predictive Value of Tests ; Prednisolone/therapeutic use ; Sella Turcica ; Thyroid Hormones/therapeutic use ; Tomography, X-Ray Computed ; Treatment Outcome

Bilateral lower motor neuron type facial palsy is an unusual neurological disorder. There are few reports that associate it with the human immunodeficiency virus (HIV) infection on initial presentation. A 51-year-old married woman, who was previously healthy and had no risk of HIV infection, presented solely with bilateral simultaneous facial palsy. A positive HIV serology test was confirmed by an enzyme-linked immunosorbent assay test. Following a short course of oral prednisolone, the patient recovered completely from facial palsy in three months, even though an antiretroviral treatment was suspended. Exclusion of HIV infection in patients with bilateral facial palsy is essential for early diagnosis and management of HIV.
Administration, Oral ; Cerebrospinal Fluid Pressure ; Enzyme-Linked Immunosorbent Assay ; Facial Paralysis ; complications ; drug therapy ; Female ; HIV Infections ; complications ; diagnosis ; Humans ; Middle Aged ; Prednisolone ; therapeutic use ; Prednisone ; administration & dosage

No abstract available.
Adult ; Antineoplastic Combined Chemotherapy Protocols/therapeutic use ; Benzamides/therapeutic use ; Bone Marrow Examination ; Chemoradiotherapy ; Cyclophosphamide/administration & dosage ; Doxorubicin/administration & dosage ; Humans ; Karyotyping ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive/*diagnosis/drug therapy/genetics/pathology ; Lymphoma, Large B-Cell, Diffuse/*diagnosis/pathology/therapy ; Male ; *Neoplasms, Second Primary ; Piperazines/therapeutic use ; Positron-Emission Tomography ; Prednisolone/administration & dosage ; Protein Kinase Inhibitors/therapeutic use ; Pyrimidines/therapeutic use ; Time Factors ; Tomography, X-Ray Computed ; Treatment Outcome ; Vincristine/administration & dosage ; Whole Body Imaging/methods

OBJECTIVETo investigate the clinical characteristics, renal pathology, treatment and prognosis of children with atypical hemolytic uremic syndrome associated with H factor antibody.

METHODFour children less than 18 yr of age admitted from Nov. 2010 to May 2011 in Peking University First Hospital were included. They all met the criteria for atypical hemolytic uremic syndrome and with positive serum anti factor H antibody. They aged from 5 to 11 yr. Data on clinical manifestations, renal pathology, treatment and prognosis were analyzed.

RESULTAll of the 4 cases had gastrointestinal symptoms such as vomiting, abdominal pain, or abdominal distension. None of them had diarrhea. Two children had hypertension. One child had episodes of convulsion. One child had history of atypical hemolytic uremic syndrome. All of them had low serum complement C3. Three of them had low serum factor H (38.0, 88.4, 209.4 mg/L). All of them had serum antibody to factor H (1: 7 068, 1: 1 110, 1: 174, and 1: 869). Three of them received renal biopsy, all of them showed thrombotic microangiopathy. All of them were treated with steroid combined with mycophenolate mofetil. Two children received plasma exchange. They were followed up for 8 to 29 months. The renal function became normal and proteinuria relieved in all of them. The serum factor H concentration increased to 405.8, 155.8 and 438.4 mg/L, respectively. The titer of anti factor H antibody decreased to 1: 119, 1: 170, 1: 123, and 1: 674, respectively.

CONCLUSIONGastrointestinal symptom is common in children with atypical hemolytic uremic syndrome associated with H factor antibody. Hypocomplementemia was observed in all of them. Steroid combined with mycophenolate mofetil seemed to be effective for them. The monitoring of serum factor H and antibody to factor H may help diagnosis and treatment.

Atypical Hemolytic Uremic Syndrome ; Autoantibodies ; blood ; immunology ; Child ; Child, Preschool ; Complement Factor H ; immunology ; Creatinine ; blood ; Female ; Hemolytic-Uremic Syndrome ; drug therapy ; immunology ; pathology ; Humans ; Kidney ; pathology ; physiopathology ; Kidney Function Tests ; Male ; Mycophenolic Acid ; administration & dosage ; analogs & derivatives ; therapeutic use ; Plasma Exchange ; Prednisolone ; administration & dosage ; therapeutic use ; Prognosis ; Retrospective Studies

INTRODUCTIONAlthough childhood autoimmune hepatitis (AIH) has been extensively investigated in the West, data on AIH in the East is lacking. We aimed to investigate AIH's clinical, biochemical and histological features, as well as its outcomes, in one of Singapore's two major paediatric units.

METHODSThis was a retrospective study of children diagnosed with AIH in the paediatric unit of National University Hospital, Singapore, over the last 12 years. Children with de novo AIH after liver transplantation were excluded. The demographic and clinical features of the patients, and their laboratory, treatment and clinical outcomes were reviewed.

RESULTSThis study comprised ten patients (six females, four males), with a median age of 5.1 (range 2.1-13.8) years at diagnosis. Five patients had inflammatory bowel disease (IBD). Seven patients had type 1 AIH, and three had autoimmune sclerosing cholangitis (ASC) and IBD; none had type 2 AIH. The median level of aspartate aminotransferase at diagnosis was 183 (range 45-2,649) U/L. Prednisolone 1 mg/kg/day was prescribed at diagnosis for eight patients. Two patients were lost to follow-up and were treated symptomatically when they re-presented with end-stage liver disease. Azathioprine or mycophenolate mofetil was prescribed after 3-7 months of treatment. Normalisation of aminotransferase levels took an average of 5.3 (range 1-39) months.

CONCLUSIONAIH is a rare but important cause of liver pathology. Children in this region with elevated aminotransferases or unexplained hepatomegaly should be screened for AIH.

Adolescent ; Aspartate Aminotransferases ; blood ; Child ; Child, Preschool ; Female ; Glucocorticoids ; administration & dosage ; Hepatitis, Autoimmune ; blood ; diagnosis ; drug therapy ; Humans ; Liver Function Tests ; Male ; Pediatrics ; Prednisolone ; administration & dosage ; Retrospective Studies ; Singapore ; Tertiary Care Centers ; Treatment Outcome